Specific Causes of Amenorrhea

Disorders of the Genital Outflow Tract and Uterus

As a cause of amenorrhea, disorders of the genital outflow tract and uterus are uncommon Congenital developmental anomalies of the genital outflow tract and uterus result from the failure of vertical fusion of vertical fusion or from failure of mullerian duct development

Imperforate Hymen

Transverse Vaginal Septum/Cervical Atresia

Mullerian Agenesis Disorders of the Genital Outflow Tract and Uterus AIS

Cervical Stenosis

Asherman Syndrome

Imperforate Hymen

Typically, patients with an imperforate hymen present with complaints of cyclic perineal, pelvis, or abdominal pressure or pain that result from cryptomenorrhea The genital examination reveals no obvious vaginal orifice and a thin, often bulging, blue perineal membrane at the inferior limit of palpable, fluctuant mass (hematocolpos)

Imperforate Hymen

The treatment of women with an imperforate hymen centers on providing relief of symptoms related to accumulated menstrual fluid and debris Definitive surgery should be accomplished as soon as possible

Transverse Vaginal Septum/Cervical Atresia

Girls with transverse vaginal septum/cervical atresia generally present with complaints of cyclic pelvis or abdominal pain Physical examination reveals a normal vaginal orifice, a shortened vagina of varying length, no visible cervix, and a palpable hematocolpos in the proximal vagina segment above the obstruction and/or a pelvic mass resulting from hematometra and hematosalpinges

Transverse Vaginal Septum/Cervical Atresia

In all instances, every effort should be made to incise and drain the sequestered menstrual fluid from below The surgical management of a transverse vaginal septum can be challenging The best surgical management for cervical atresia is controversial

Mullerian Agenesis

Patients with mullerian agenesis exhibit normal, symmetrical breast and pubic hair development, no visible vagina, and have no symptoms or signs of cryptomenorrhea because the rudimentary uteri contain no functional endometrium

Two form of the disorder have been describes Type A and Type B

Mullerian Agenesis

Progressive vaginal dilatation appropriate and effective first choice creation of a functional vagina Operative treatment of mullerian agenesis generally can be reserved for those who are unable or unwilling to dedicate themselves to a program of progressive vaginal dilatation and for those in whom earnest efforts fail MacIndoe procedure and Vecchietti operation

Androgen Insensitivity Syndrome

Patients with complete AIS exhibit asymmetrical secondary sexual development, a short vagina with no visible cervix, and have no other symptoms or complaints Patients with incomplete AIS have a greater sensitivity to androgen pubic hair growth may accompany breast development and the clitoris may enlarge

Androgen Insensitivity Syndrome

The treatment of patients with complete AIS has two major components, one focusing on creation of a functional vagina, and another relating to the risk for developing malignancy in the cryptorchid testes Complete AIS is the only exception to the rule that gonads with a Y chromosome should be removed as soon as diagnosis is made

Cervical Stenosis

Severe cervical stenosis with complete outflow obstruction is a rare complication of cervical conization procedure or other surgical treatments for CIN The treatment for cervical stenosis is careful dilatation

Asherman Syndrome

Although Asherman Syndrome from any cause may result in amenorrhea, most women with intrauterine adhesions present with dysmenorrhea, hypomenorrhea, infertility, or recurrent pregnancy loss, rather than amenorrhea

Asherman Syndrome

Operative hysterescopy is the primary method for treatment of intrauterine adhesions that may be lysed by scissors, electrodissections, or with a laser

Disorders of the Ovary

Disorders of the ovary include the most common causes of amenorrhea and can present as primary or secondary amenorrhea

Gonadal Dysgenesis

The most common causes of primary amenorrhea By far, the most common form of gonadal dysgenesis is Turner Syndrome

Turner Syndrome

Patients with Turner Syndrome generally present with primary amenorrhea and absent secondary sexual development Approximately 15% of patients with Turner Syndrome begin but do not complete pubertal development and approximately 5% complete puberty and begin menstruation

Turner Syndrome

Treatment with estrogen must be timed carefully, with goals of minimizing its adverse effects and inducing puberty at an approximately normal age the goal of completing sexual maturation over a period of 2-3 years

Swyer Syndrome (46,XY Gonadal Dysgenesis)

Patients with Swyer Syndrome generally present with delayed sexual maturation and primary amenorrhea Gonadectomy is indicated soon after diagnosis due to the significant risk for malignant transformation in occult testicular elements The same sequential sex steroid treatment regimen for patients with Turner Syndrome can be applied for patients with Swyer syndrome

46, XX Gonadal Dysgenesis

Some individuals with primary amenorrhea and gonadal dysgenesis have a normal 46,XX karyotype autosomal genes also play a critical role in ovarian differentiation

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